Idiopathic pulmonary fibrosis: an epithelial/fibroblastic cross-talk disorder

Respiratory Research

Annie Pardo¹  Moisés Selman¹ 

[1] Instituto Nacional de Enfermedades Respiratorias & Facultad de Ciencias, UNAM, México DF, México

关键词: pulmonary fibrosis;    myofibroblasts;    extracellular matrix;    epithelial cells;    apoptosis;   

Others  :  1227418
DOI  :  10.1186/rr175

 received in 2001-07-26, accepted in 2001-08-21,  发布年份 2001

【 摘 要 】

Idiopathic pulmonary fibrosis is a chronic and usually progressive lung disorder of unknown etiology. A growing body of evidence suggests that, in contrast to other interstitial lung diseases, IPF is a distinct entity in which inflammation is a secondary and non-relevant pathogenic partner. Evidence includes the presence of similar mild/moderate inflammation either in early or late disease, and the lack of response to potent anti-inflammatory therapy. Additionally, it is clear from experimental models and some human diseases that it is possible to have fibrosis without inflammation. An evolving hypothesis proposes that IPF may result from epithelial micro-injuries and abnormal wound healing.

【 授权许可】

   
2001 BioMed Central Ltd

【 图 表 】

Figure 1.

Figure 2.

【 参考文献 】

• [1]International consensus statement: Idiopathic pulmonary fibrosis: diagnosis and treatment. Am J Respir Crit Care Med 2000, 161:646-664.
• [2]Perez-Padilla R, Salas J, Chapela R, Sanchez M, Carrillo G, Perez R, Gaxiola M, Selman M: Mortality in Mexican patients with chronic pigeon breeder's lung compared with those with usual interstitial pneumonia. Am Rev Respir Dis 1993, 148:49-53.
• [3]Bjoraker JA, Ryu JH, Edwin MK, Myers JL, Tazelaar HD, Schroeder DR, Offord KP: Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 1998, 157:199-203.
• [4]Douglas WW, Ryu JH, Schroeder DR: Idiopathic pulmonary fibrosis. Impact of oxygen and colchicine, prednisone, or no therapy on survival. Am J Respir Crit Care Med 2000, 161:1172-1178.
• [5]Selman M, King TE, Pardo A: Idiopathic pulmonary fibrosis: prevaling and evolving hypotheses about its pathogenesis and implications for therapy. Ann Intern Med 2001, 134:136-151.
• [6]Katzenstein ALA, Fiorelli RF: Nonspecific interstitial pneumonia/fibrosis. Histologic features and clinical significance. Am J Surg Pathol 1994, 18:136-147.
• [7]Borzone G, Moreno R, Urrea R, Meneses M, Oyarzun M, Lisboa C: Bleomycin-induced chronic lung damage does not resemble human idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2001, 163:1648-1653.
• [8]Nicholson AG, Colby TV, du Bois RM, Hansell DM, Wells AU: The prognostic significance of the histologic pattern of interstitial pneumonia in patients presenting with the clinical entity of cryptogenic fibrosing alveolitis. Am J Respir Crit Care Med 2000, 162:2213-2217.
• [9]Katzenstein ALA, Myers JL: Idiopathic pulmonary fibrosis. Clinical relevance of pathologic classification. Am J Respir Crit Care Med 1998, 157:1301-1315.
• [10]King TE, Sdchwarz MI, Brown K, Tose JA, Colby TV, Waldron JA, Flint A, Thurlbeck W, Cherniack RM: Extent of fibroblastic foci predict mortality in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2001, 163:A982.
• [11]Adamson IYR, Young L, Bowden DH: Relationship of alveolar epithelial injury and repair to the induction of pulmonary fibrosis. Am J Pathol 1988, 130:377-383.
• [12]Regan W, Wold LE, Coonrad R, Morrey BF: Microscopic histopathology of chronic refractory lateral epicondylitis. Am J Sports Med 1992, 20:746-749.
• [13]Kraushaar BS, Nirschl RP: Tendinosis of the elbow (tennis elbow): clinical features and findings of histological, immunohistochemical, and electron microscopy studies. J Bone Joint Surg Am 1999, 81:259-279.
• [14]Kuhn C III, Boldt J, King TE Jr, Crouch E, Vartio T, McDonald JA: An immunohistochemical study of architectural remodeling and connective tissue synthesis in pulmonary fibrosis. Am Rev Respir Dis 1989, 140:1693-1703.
• [15]Kasper M, Haroske G: Alterations in the alveolar epithelium after injury leading to pulmonary fibrosis. Histol Histopathol 1996, 11:463-483.
• [16]Kapanci Y, Desmouliere A, Pache JC, Redard M, Gabbiani G: Cytoskeletal protein modulation in pulmonary alveolar myofibroblasts during idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 1995, 152:2163-2169.
• [17]Nash JRG, McLaughlin PJ, Butcher D, Corrin B: Expression of tumour necrosis factor-α in cryptogenic fibrosing alveolitis. Histopathology 1993, 22:343-347.
• [18]Khalil N, O'Connor RN, Unruh HW, Warren PW, Flanders KC, Kemp A, Bereznay OH, Greenberg AH: Increased production and immunohistochemical localization of transforming growth factor-β in idiopathic pulmonary fibrosis. Am J Respir Cell Mol Biol 1991, 5:155-162.
• [19]Khalil N, O'Connor RN, Flanders KC, Unruh HW: TGF-β1, but not TGF-β2 or TGF-β3, is differentially present in epithelial cells of advanced pulmonary fibrosis: an immunohistochemical study. Am J Respir Cell Mol Biol 1996, 14:131-138.
• [20]Antoniades NH, Bravo M, Avila R, Galanopoulus T, Neville J, Selman M: Platelet-derived growth factor in idiopathic pulmonary fibrosis. J Clin Invest 1990, 86:1055-1064.
• [21]Kuhn C, McDonald JA: The roles of the myofibroblast in idiopathic pulmonary fibrosis. Ultrastructural and immunohistochemical features of sites of active extracellular matrix synthesis. Am J Pathol 1991, 138:1257-1265.
• [22]Fukuda Y, Basset F, Ferrans VJ, Yamanaka N: Significance of early intra-alveolar fibrotic lesions and integrin expression in lung biopsy specimens from patients with idiopathic pulmonary fibrosis. Hum Pathol 1995, 26:53-61.
• [23]Uhal BD, Joshi I, True AL, Mundle S, Raza A, Pardo A, Selman M: Fibroblasts isolated after fibrotic lung injury induce apoptosis of alveolar epithelial cells in vitro. Am J Physiol 1995, 269:L819-828.
• [24]Wang R, Ramos C, Joshi I, Zagariya A, Pardo A, Selman M, Uhal B: Human lung myofibroblast-derived inducers of alveolar epithelial apoptosis identified as angiotensin peptides. Am J Physiol 1999, 277:L1158-L1164.
• [25]Uhal BD, Joshi I, Hughes WF, Ramos C, Pardo A, Selman M: Alveolar epithelial cell death adjacent to underlying myofibroblasts in advanced fibrotic human lung. Am J Physiol 1998, 275:L1192-L1199.
• [26]Barbas-Filho JV, Ferreira MA, Sesso A, Kairalla RA, Carvalho CR, Capelozzi VL: Evidence of type II pneumocyte apoptosis in the pathogenesis of idiopathic pulmonary fibrosis (IFP)/usual interstitial pneumonia (UIP). J Clin Pathol 2001, 54:132-138.
• [27]Kuwano K, Miyazaki H, Hagimoto N, Kawasaki M, Fujita M, Kuni-take R, Kaneko Y, Hara N: The involvement of Fas-Fas ligand pathway in fibrosing lung diseases. Am J Respir Cell Mol Biol 1999, 20:53-60.
• [28]Maeyama T, Kuwano K, Kawasaki M, Kunitake R, Hagimoto N, Matsuba T, Yoshimi M, Inoshima I, Yoshida K, Hara N: Upregulation of Fas-signalling molecules in lung epithelial cells from patients with idiopathic pulmonary fibrosis. Eur Respir J 2001, 17:180-189.
• [29]Ehrlich HP, Desmouliere A, Diegelmann RF, Cohen IK, Compton CC, Garner WL, Kapanci Y, Gabbiani G: Morphological and immunochemical differences between keloid and hypertrophic scar. Am J Pathol 1994, 145:105-113.
• [30]Chodon T, Sugihara T, Igawa HH, Funayama E, Furukawa H: Keloid-derived fibroblasts are refractory to Fas-mediated apoptosis and neutralization of autocrine transforming growth factor-beta 1 can abrogate this resistance. Am J Pathol 2000, 157:1661-1669.
• [31]Luo S, Benathan M, Raffoul W, Panizzon RG, Egloff DV: Abnormal balance between proliferation and apoptotic cell death in fibroblasts derived from keloid lesions. Plast Reconstr Surg 2001, 107:87-96.
• [32]Lappi-Blanco E, Soini Y, Paakko P: Apoptotic activity is increased in the newly formed fibromyxoid connective tissue in bronchiolitis obliterans organizing pneumonia. Lung 1999, 177:367-376.
• [33]Ramos C, Montaño M, García-Alvarez J, Ruiz V, Uhal BD, Selman M, Pardo A: Fibroblasts from idiopathic pulmonary fibrosis and normal lungs differ in growth rate, apoptosis, and TIMPs expresion. Am J Respir Cell Mol Biol 2001, 24:591-598.
• [34]Jelaska A, Korn JH: Role of apoptosis and transforming growth factor beta1 in fibroblast selection and activation in systemic sclerosis. Arthritis Rheum 2000, 43:2230-2239.
• [35]Jordana M, Schulman J, McSharry C, Irving LB, Newhouse MT, Jordana G, Gauldie J: Heterogeneous proliferative characteristics of human adult lung fibroblast lines and clonally derived fibroblasts from control and fibrotic tissue. Am Rev Respir Dis 1988, 137:579-584.
• [36]Raghu G, Chen YY, Rusch V, Rabinovitch PS: Differential proliferation of fibroblasts cultured from normal and fibrotic human Lung. Am Rev Respir Dis 1988, 138:703-708.
• [37]Legrand C, Polette M, Tournier JM, de Bentzmann S, Huet E, Monteau M, Birembaut P: UPa/plasmin system-mediated MMP-9 activation is implicated in bronchial epithelial cell migration. Exp Cell Res 2001, 264:326-336.
• [38]Davis G, Pintar Allen K, Salazar R, Maxwell S: Matrix metalloproteinase-1 and -9 activation by plasmin regulates a novel endothelial cell-mediated mechanism of collagen gel contraction and capillary tube regression in three-dimensional collagen matrices. J Cell Sci 2001, 114:917-930.
• [39]Kotani I, Sato A, Hayakawa H, Urano T, Takada Y, Takada A: Increased procoagulant and antifibrinolytic activities in the lungs with idiopathic pulmonary fibrosis. Thromb Res 1995, 77:493-504.
• [40]Imokawa S, Sato A, Hayakawa H, Kotani M, Urano T, Takada A: Tissue factor expression and fibrin deposition in the lungs of patients with idiopathic pulmonary fibrosis and systemic sclerosis. Am J Respir Crit Care Med 1997, 156:631-636.
• [41]Fujii M, Hayakawa H, Urano T: Relevance of tissue factor and tissue factor pathway inhibitor for hypercoagulable state in the lungs of patients with idiopathic pulmonary fibrosis. Thromb Res 2000, 99:111-117.
• [42]Saito Y, Suzuki E, Moriyama H, Terada M, Hasegawa T, Ooi H, Kobayashi Y, Tsuchiya T, Geivo F: Decreased ability to generate plasmin in lung tissue of usual interstitial pneumonia compared with bronchiolitis obliterans organizing pneumonia. Am J Respir Crit Care Med 2000, 161:A826.
• [43]Higgins PJ, Slack JK, Diegelmann RF, Staiano-Coico L: Differential regulation of PAI-1 gene expression in human fibroblasts predisposed to a fibrotic phenotype. Exp Cell Res 1999, 248:634-642.
• [44]Hayashi T, Stetler-Stevenson WG, Fleming MV, Fishback N, Koss MN, Liotta LA, Ferrans VJ, Travis WD: Immunohistochemical study of metalloproteinases and their tissue inhibitors in the lungs of patients with diffuse alveolar damage and idiopathic pulmonary fibrosis. Am J Pathol 1996, 149:1241-1256.
• [45]Fukuda Y, Ishizaki M, Kudoh S, Kitaichi M, Yamanaka N: Localization of matrix metalloproteinases-1, -2, and -9, and tissue inhibitor of metalloproteinase-2 in interstitial lung diseases. Lab Invest 1998, 78:687-698.
• [46]Selman M, Ruiz V, Cabrera S, Segura L, Ramírez R, Barrios R, Pardo A: TIMP 1, 2, 3 and 4 in idiopathic pulmonary fibrosis. A prevailing non-degradative lung microenvironment? Am J Physiol 2000, 279:L562-L574.
• [47]Zhang K, Rekhter MD, Gordon D, Phan SH: Myofibroblasts and their role in lung collagen gene expression during pulmonary fibrosis. Am J Pathol 1994, 145:114-125.
• [48]Parks WC, Sudbeck BD, Doyle GR, Saariahlo-Kere UK: Matrix metalloproteinases in tissue repair. In Matrix Metalloproteinases. Edited by Parks WC, Mecham RP. San Diego: Academic Press; 1998, 263-297.